Saudi Journal of Ophthalmology (2016) 30, 144–147

Case Report

Kyrieleis plaques associated with Herpes Simplex Virus type 1acute retinal necrosis

Peer review under responsibilityof Saudi Ophthalmological Society,King Saud University Production and hosting by Elsevier

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Received 8 May 2015; received in revised form 20 January 2016; accepted 11 February 2016; available online 18 February 2016.

ICARE Eye Hospital and Postgraduate Institute, Noida, U.P., India

⇑ Corresponding author at: 57, Mayur Vihar Phase 1 Extension, New Delhi 110091, India. Tel.: +91 9811179191; fax: +91 11 23230033.e-mail address: nehadoc@hotmail.com (N. Goel).

Neha Goel ⇑; Amrita Sawhney

Abstract

We report the case of a 55-year-old immunocompetent male who presented with features typical of acute retinal necrosis (ARN).Polymerase chain reaction of the aqueous tap was positive for Herpes Simplex Virus (HSV) – 1. Following therapy with intravenousAcyclovir, followed by oral Acyclovir and steroids, there was marked improvement in the visual acuity and clinical picture. At oneweek after initiation of treatment, Kyrieleis plaques were observed in the retinal arteries. They became more prominent despiteresolution of the vitritis, retinal necrosis and vasculitis and persisted till six weeks of follow-up, when fluorescein angiography wasperformed. The appearance of this segmental retinal periarteritis also known as Kyrieleis plaques has not been described in ARNdue to HSV-1 earlier.

Keywords: Kyrieleis plaque, Acute retinal necrosis, Segmental periarteritis, Herpes Simplex Virus type 1

� 2016 The Authors. Production and hosting by Elsevier B.V. on behalf of Saudi Ophthalmological Society, King Saud University.This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

http://dx.doi.org/10.1016/j.sjopt.2016.02.005

Introduction

Kyrieleis plaques are a rarely encountered clinical entity inwhich whitish segmented deposits are seen scattered alongretinal arterial branches in a beaded pattern.1 Also knownas segmental periarteritis,2 this feature has primarily beendescribed in association with toxoplasmosis,3 tuberculosis,1

syphilis4 and Mediterranean spotted fever.5 We present theoccurrence and course of Kyrieleis plaques in acute retinalnecrosis (ARN) due to Herpes Simplex Virus (HSV) – 1.

Case report

A 55-year-old immunocompetent male presented withdecreased vision and floaters in his left eye since 10 days.There was no significant past medical history. Best correctedvisual acuity (BCVA) was 20/20 in the right eye and 20/200 inthe left eye. Slit lamp examination demonstrated granuloma-

tous anterior uveitis with 3+ cells in the anterior chamber inthe affected eye. A dilated fundus examination of this eyerevealed 2+ vitreous cells and patches of retinitis in the midand far retinal periphery with neighboring vasculitis (Fig. 1).The right eye examination was unremarkable.

Polymerase chain reaction of the aqueous tap was positivefor HSV-1 and negative for HSV-2, Varicella Zoster Virus (VZV)and Cytomegalovirus (CMV). A diagnosis of unilateral ARNwas made. The patient was started on intravenous Acyclovir500 mg three times a day for 7 days followed by oralAcyclovir 800 mg five times a day with oral Prednisone60 mg daily. Topical steroids and cycloplegics were alsoadministered in the left eye.

The patient demonstrated improvement within a week ofinitiation of therapy. At this stage, Kyrieleis plaques wereobserved along two inferior retinal arteries (Fig. 2a and d).At two weeks follow-up, BCVA improved to 20/60 with reso-lution of the anterior uveitis and decrease in the vitritis and

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Figure 1. Colour fundus photograph of the left eye at presentation showing vitritis (a), peripheral areas of retinal necrosis with neighboring vasculitis inthe temporal (b), inferior (c) and nasal quadrants (d).

Figure 2. Weekly fundus photographs of the left eye following initiation of therapy. (a) After a week of intravenous Acyclovir, vitritis decreased andKyrieleis plaques were visible in the inferior retinal arteries (arrows). (d) The borders of the retinal necrotic lesions became more well defined. The patientwas shifted to oral Acyclovir and steroids. (b) A week later, there was further decrease in the vitritis and the retinal necrosis (e). Treatment was continuedand at three weeks, there was resolution of the vitritis (c) and peripheral retinal necrosis (f). Kyrieleis plaques were seen in nasal retinal arteries as well, asyellowish plaques that did not extend beyond the vessel walls (arrows). There was no involvement of the retinal veins.

Kyrieleis plaques in acute retinal necrosis 145

peripheral retinal necrosis (Fig. 2b and e). Three weeks later,BCVA was 20/30, vitritis had resolved and the retinitis was nolonger active. At this stage, Kyrieleis plaques were morenumerous and prominent, also observed along nasal retinalarteries (Fig. 2c and f). These persisted till six weeks follow-up (Fig. 3a), when fluorescein angiography was performed.There was no delay in arterial filling, no leakage from the reti-nal arterioles and the plaques themselves did not fluoresce(Fig. 3b and c). Staining was present in the areas of resolved

retinitis (Fig. 3d). Steroids were tapered and oral Acyclovirdiscontinued after two weeks.

Discussion

ARN is an uncommon intraocular inflammatory syndromethat typically affects immunocompetent individuals of allage groups. Clinically, it is characterized by anterior uveitis,dense vitritis, progressive retinal necrosis that begins in the

Figure 3. (a) At six weeks following presentation, fundus photograph of the left eye showing Kyrieleis plaques in the retinal arteries (arrows). Fundusfluorescein angiography did not demonstrate any leakage or staining of the retinal arteries in the areas of the plaques (b and c). The peripheral healednecrotic retinal areas showed staining (d).

146 N. Goel, A. Sawhney

periphery and occlusive vasculitis. The diagnostic criteria forARN were established by the American Uveitis Society in1994.6 ARN usually presents unilaterally and carries a poorprognosis. Visual loss occurs due to retinal detachment, opticneuropathy and ischemic vasculopathy involving the macula.7

The etiological agents of ARN include DNA viruses such asVZV, HSV and less commonly CMV. While HSV-2 is com-moner in younger patients, HSV-1 or VZV is more prevalentin older patients.7 These can be identified by PCR-basedassays of ocular fluids. However, ARN is principally a clinicaldiagnosis and institution of therapy should not be delayedwhile awaiting laboratory confirmation. Prompt diagnosisand treatment is essential to salvage vision in this frequentlyblinding condition and preventing involvement of the felloweye. Treatment involves extended use of antiviral agents.Systemic steroids help to limit damage caused by the severeinflammation associated with ARN.7

Kyrieleis plaques were initially described in an eye withtuberculous uveitis by Kyrieleis in 1933.1 The term ‘‘segmen-tal periarteritis’’ was used later to describe these whitish seg-mented deposits found within retinal arteries.2 Kyrieleisplaques have also been associated with infections of theretina due to toxoplasma, syphilis and Rickettsia conorii.3–5

Their occurrence in ARN is rare. There is a single case reportof Kyrieleis plaques in ARN due to HSV-2,8 and two reports inARN due to VZV.9,10 To the best of our knowledge, this is thefirst report of Kyrieleis plaques in ARN due to HSV-1.

Kyrieleis plaques can be differentiated from vascularsheathing and frosted branch angiitis. While the formeraffects retinal arteries exclusively, the latter can involve both

retinal arteries and veins. Also, the plaques do not leak fluo-rescein as opposed to frosted branch angiitis.11

Since there is no pathological study of Kyrieleis plaques,their nature is still unknown. In our patient, these plaquesappeared after one week of therapy, when the vitritis andretinitis were resolved and increased thereafter. A similarcourse has been observed in the previous cases.9,10 This sug-gests that they may represent an immunological response toan infectious agent resulting in the deposition of inflamma-tory debris within or adjacent to the vessel wall.12 However,persistence of the plaques despite resolution of the infectionand treatment with steroids contradicts this hypothesis.3

In summary, this case represents a typical ARN syndromedue to HSV-1 infection that demonstrated Kyrieleis plaquesfollowing treatment. Occurrence of Kyrieleis plaques maybe more common than the literature suggests, and theymay be underreported. Our case adds another cause to theetiology of these plaques.

Conflict of interest

The authors declared that there is no conflict of interest.

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